Cardiopulmonary phenotype in systemic sclerosis associated pulmonary hypertension

Introduction

Pulmonary hypertension (PH) associated with systemic sclerosis (SSc) increases morbidity and mortality. Cardiopulmonary comorbidities, as per the 2021 PH consensus, play a role in the choice of therapy between monotherapy and combination therapy.

Methods

A cross-sectional study was conducted in patients with SSc based on the 2013 ACR/EULAR criteria or very early disease (VEDOSS 2011). PH was considered if they met the following criteria: pulmonary artery systolic pressure (PASP) > 39 mmHg or peak tricuspid regurgitation velocity (PTRV) > 3.4 m/s, PASP between 33 and 39 mmHg or PTRV between 2.9 and 3.4 m/s plus two additional findings suggestive of PH. PH was classified as type 2 if LVEF < 50% or moderate to severe diastolic dysfunction was present; type 3 if extensive interstitial disease on tomography > 20% or forced vital capacity (FVC) < 75%; type 4 if abnormalities related to embolism were detected on scintigraphy or tomography. If patients did not meet these criteria, they were classified as type 1 PH. Complete data on cardiopulmonary risk factors and other factors were required. The frequency of these factors in the population and differences between groups based on risk factors were estimated.

Results

A total of 228 patients were selected. Three had type 2 PH, 24 had type 3, and 40 had type 1 PH, with the majority (75%) having at least one cardiopulmonary risk factor, and 47.5% having more than one. Mild diastolic dysfunction (25%) and hypertension (35%) were the most prevalent. In the type 1 PH group, those with risk factors experienced an increase in the number of years with Raynaud's phenomenon, anticentromere antibodies, and gastrointestinal symptoms (p < 0.05).

Conclusion

In patients with PH, 75% have one, and 45% have two or more risk factors.